Research

SnapShot: Interactions between B Cells and T Cells.

Cell. 2015 Aug 13;162(4):926-926.e1

Authors: Tangye SG, Brink R, Goodnow CC, Phan TG

Abstract
Dynamic interactions between B and T cells underpin the development of adaptive humoral immune responses to infections and vaccines. Recent advances in the molecular and spatiotemporal control of these interactions during primary responses have contributed greatly to elucidating the molecular pathogenesis of numerous immunodeficiency and autoimmune diseases. The next challenge is to determine how and where memory B and T cells interact during secondary responses to facilitate the rapid and robust response that characterizes anamnestic immunity.

PMID: 26276638 [PubMed – in process]

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Adverse reactions to Mycobacterium bovis bacille Calmette-Guérin vaccination against tuberculosis in Iranian children.

Clin Exp Vaccine Res. 2015 Jul;4(2):195-9

Authors: Mahmoudi S, Khaheshi S, Pourakbari B, Aghamohammadi A, Keshavarz Valian S, Bahador A, Sabouni F, Ramezani A, Mamishi S

Abstract
PURPOSE: There are considerable variations in the number of adverse reaction reports related to vaccine from different countries. The aim of this study was to review the development of adverse reactions to bacille Calmette-Guérin (BCG) vaccination among hospitalized patients in an Iranian referral hospital.
MATERIALS AND METHODS: We identified hospitalized patients with BCG complications in Pediatric Infectious Disease Research Center, Tehran University of Medical Sciences, Tehran, Iran during January 2007-April 2009. Data on demographics, clinical features, laboratory findings, personal history (including vaccination history), family history, and outcomes were retrieved from medical records.
RESULTS: There were 46 cases with BCG complication during the 2 years period. All of the children received vaccination at birth. Twenty-eight patients (61%) were male. The mean age of the patients was 13.5 ±11.3 months (range, 1 to 52 months; median, 10 months). The majority of children (57%) with BCG complication were less than 1 year old. Among hospitalized patients due to BCG complications, suppurative lymphadenitis was occurred in 28 children (61%) and lymphadenopathy was seen in 9 children (20%). Disseminated BCG was detected in 8 patients (17%) and only 1 child (2%) was presented with abscess. In 7% (n = 3) of children, the family history of BCG complications were positive.
CONCLUSION: The most common side effect of the BCG vaccine in our study was suppurative lymphadenitis. Disseminated BCG infection in complications leading to hospitalization in our study was 17%. With regard to the difficulty in implementing such a guideline in settings where BCG is given to all newborns, registration of Iranian primary immunodeficiency disorders (PID) patients would be helpful to increase the awareness of medical community of Iran to investigate underlying disease. In addition, BCG vaccination should postpone in each newborn with a family history of PID until the definite condition has been ruled out.

PMID: 26273579 [PubMed]

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Different brands of intravenous immunoglobulin for primary immunodeficiencies: how to choose the best option for the patient?

Expert Rev Clin Immunol. 2015 Aug 14;:1-15

Authors: Abolhassani H, Asgardoon MH, Rezaei N, Hammarstrom L, Aghamohammadi A

Abstract
Immunoglobulin (IG) replacement therapy has served as lifesaving treatment in primary immunodeficiency diseases (PID) for more than six decades. Approximately 70% of patients with PID require IG replacement to maintain their health during the course of disease. It is estimated that about one-third of IG products is used for replacement therapy in these patients. On the other hand, the introduction of newer IG preparations is continuing to improve and extend the quality of life in PID patients. Because of the options available including concentrations, formulations, osmolality, product stabilizers, sodium concentration, anti-infective activity, IgA content and pH, it is important to match the PID patient’s complications with potential side effects associated with the composition of a particular IG product. The purpose of this review is to present the clinical differences of intravenous IG among the various preparations regarding PID patients.

PMID: 26272002 [PubMed – as supplied by publisher]

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Validity of Primary Immunodeficiency Disease Diagnoses in United States Medicaid Data.

J Clin Immunol. 2015 Aug 14;

Authors: Hernandez-Trujillo H, Orange JS, Roy JA, Wang Y, Newcomb CN, Liu Q, Hennessy S, Lo Re V

Abstract
PURPOSE: Primary immunodeficiency diseases (PID) are a rare group of disorders with a wide array of clinical presentations. The absence of validated methods to identify these diseases in electronic databases has limited understanding of their epidemiology and the impact of drug therapies on outcomes. We measured the positive predictive values (PPVs) of ICD-9 diagnoses for identifying PID within US Medicaid.
METHODS: We identified Medicaid patients from California, Florida, New York, Ohio, and Pennsylvania with PID ICD-9 diagnoses (common variable immunodeficiency [279.06], X-linked agammaglobulinemia [279.04], hyper-immunoglobulin M syndrome [279.05], Wiskott Aldrich Syndrome [279.12]) recorded at least twice from 1999 to 2007. Outpatient records were reviewed by a clinical immunologist to adjudicate diagnoses. PPVs with 95 % confidence intervals (CIs) for confirmed outcomes were determined for individual ICD-9 diagnoses and combinations of diagnoses and Current Procedural Terminology codes for a quantitative immunoglobulin test (82784) or immunoglobulin infusion (96365).
RESULTS: Among 83 patients with PID ICD-9 diagnoses, 16 were adjudicated as having the condition (PPV, 19.3 %; 95 % CI, 11.4-29.4 %). Individual ICD-9 diagnoses had low PPVs (range, 16.7-33.3 %). Requiring procedural codes for quantitative immunoglobulins or intravenous immunoglobulin did not increase PPVs of these diagnoses (range, 11.1-41.7 %). An X-linked agammaglobulinemia diagnosis plus intravenous immunoglobulin had the highest PPV among the algorithms evaluated (PPV, 41.7 %; 95 % CI, 15.1-72.3 %).
CONCLUSIONS: Algorithms comprising PID ICD-9 diagnoses and procedures for quantitative immunoglobulin tests and immunoglobulin infusion had low PPVs for adjudicated diagnoses in Medicaid. Alternative data sources should be evaluated to study the epidemiology of these diseases.

PMID: 26271389 [PubMed – as supplied by publisher]

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Primary Diffuse Large B-cell Lymphoma Arising in the Tongue Accompanied by Ataxia-telangiectasia: A Case Report.

J Clin Diagn Res. 2015 Jun;9(6):ZD25-7

Authors: Tomioka H, Kaneoya A, Mochizuki Y, Harada H

Abstract
Ataxia-telangiectasia (AT) is a rare autosomal recessive disorder that is characterized by progressive cerebellar ataxia, telangiectasia, immunodeficiency, and a predisposition to leukemia/lymphoma. Here we report a rare case of lymphoma of the tongue accompanied by AT. Tumour extirpation was performed and diffuse large B-cell lymphoma was diagnosed following pathologic examination. A whole-body survey showed no other enlarged lymph nodes or tumour. The female patient then received a modified dosage of COPAD (cyclophosphamide, vinblastine, pirarubicin, and prednisolone) plus rituximab to avoid severe complications. As of follow-up after 3 years and 5 months, she remains in complete remission. Patients showing AT need careful surveillance and long-term continuous follow-up.

PMID: 26266230 [PubMed]

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Rare gastrointestinal lymphomas: The endoscopic investigation.

World J Gastrointest Endosc. 2015 Aug 10;7(10):928-49

Authors: Vetro C, Bonanno G, Giulietti G, Romano A, Conticello C, Chiarenza A, Spina P, Coppolino F, Cunsolo R, Raimondo FD

Abstract
Gastrointestinal lymphomas represent up to 10% of gastrointestinal malignancies and about one third of non-Hodgkin lymphomas. The most prominent histologies are mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma. However, the gastrointestinal tract can be the site of rarer lymphoma subtypes as a primary or secondary localization. Due to their rarity and the multifaceted histology, an endoscopic classification has not been validated yet. This review aims to analyze the endoscopic presentation of rare gastrointestinal lymphomas from disease diagnosis to follow-up, according to the involved site and lymphoma subtype. Existing, new and emerging endoscopic technologies have been examined. In particular, we investigated the diagnostic, prognostic and follow-up endoscopic features of T-cell and natural killer lymphomas, lymphomatous polyposis and mantle cell lymphoma, follicular lymphoma, plasma cell related disease, gastrointestinal lymphomas in immunodeficiency and Hodgkin’s lymphoma of the gastrointestinal tract. Contrarily to more frequent gastrointestinal lymphomas, data about rare lymphomas are mostly extracted from case series and case reports. Due to the data paucity, a synergism between gastroenterologists and hematologists is required in order to better manage the disease. Indeed, clinical and prognostic features are different from nodal and extranodal or the bone marrow (in case of plasma cell disease) counterpart. Therefore, the approach should be based on the knowledge of the peculiar behavior and natural history of disease.

PMID: 26265987 [PubMed]

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Related Articles

Zinc-binding metallothioneins are key modulators of IL-4 production by basophils.

Mol Immunol. 2015 Aug;66(2):180-8

Authors: Ugajin T, Nishida K, Yamasaki S, Suzuki J, Mita M, Kubo M, Yokozeki H, Hirano T

Abstract
Zinc (Zn) is an essential nutrient, and Zn deficiency causes immunodeficiency and skin disorders. Basophils express FcɛRI on their surface and release multiple mediators after receptor cross-linking, including large amounts of IL-4. However, the mechanisms involved in the FcɛRI-mediated regulation of basophil IL-4 production are currently unclear. Here, we show that the Zn-binding metallothionein (MT) proteins are essential for the FcɛRI-induced basophil production of IL-4. Basophils from MT-I/II(-/-) mice produced significantly less FcɛRI-induced IL-4 than did wild-type basophils. The MTs were involved in maintaining intracellular Zn levels, thereby regulated the calcineurin activity and nuclear factor of activated T-cell (NFAT)-mediated IL-4 production. These results suggest that the MT-dependent control of Zn homeostasis is a novel mechanism for regulating basophil IL-4 production.

PMID: 25801306 [PubMed – indexed for MEDLINE]

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Primary Adrenal Lymphoma Possibly Associated With Epstein-Barr Virus Reactivation Due to Immunosuppression Under Methotrexate Therapy.

Medicine (Baltimore). 2015 Aug;94(31):e1270

Authors: Ohkura Y, Shindoh J, Haruta S, Kaji D, Ota Y, Fujii T, Hashimoto M, Watanabe G, Matsuda M

Abstract
Primary adrenal lymphoproliferative disorder (LPD) is an extremely rare disease that is widely known to be associated with methotrexate (MTX) use in patients with rheumatoid arthritis (RA).A 70-year-old man was incidentally found to have a tumor at the dorsal part of the liver in a medical check-up. He had a history of RA treated with MTX. Abdominal ultrasonography demonstrated a low echoic mass (30 mm in diameter) at the dorsal part of the liver, located close to the inferior vena cava. Preoperative differential diagnoses included intrahepatic cholangiocarcinoma, adrenal tumor, and hepatic malignant lymphoma, but no definitive diagnosis was reached. On exploratory laparotomy, the tumor seemed to be derived from the right adrenal gland and adhered tightly to segment 7 of the liver. Therefore, right adrenectomy with partial resection of segment 7 of the liver was performed. Pathological findings revealed diffuse inflammatory cell infiltration with a population of small atypical lymphoid cells, with positive immunohistochemical evidence for Epstein-Barr virus (EBV). Final diagnosis was primary adrenal iatrogenic EBV-positive LPD, classified as “other iatrogenic immunodeficiency-associated LPDs: Hodgkin-like lesions.”In this report, we described the possibility of the spontaneous healing of MTX-associated LPD (MTX-LPD) before treatment and the importance of doubting MTX-LPD and doing immunostaining to necrotic tissue. To our knowledge, this is the first reported case of MTX-related EBV-positive LPD, Hodgkin-like lesion, of the unilateral adrenal gland in patient with RA.

PMID: 26252293 [PubMed – as supplied by publisher]

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[Primary immunodeficiency association with systemic lupus erythematosus: review of literature and lessons learned by the Rheumatology Division of a tertiary University Hospital at São Paulo].

Rev Bras Reumatol. 2015 Jul 16;

Authors: Errante PR, Perazzio SF, Frazão JB, Silva NP, Andrade LE

Abstract
Primary immunodeficiency disorders (PID) represent a heterogeneous group of diseases resulting from inherited defects in the development, maturation and normal function of immune cells; thus, make individuals susceptible to recurrent infections, allergy, autoimmunity, and malignancies. In this retrospective study, autoimmune diseases (AIDs), in special systemic lupus erythematosus (SLE) which arose associated to the course of PID, are described. Classically, the literature describes three groups of PID associated with SLE: (1) deficiency of Complement pathway components, (2) defects in immunoglobulin synthesis, and (3) chronic granulomatous disease (CGD). Currently, other PID have been described with clinical manifestation of SLE, such as Wiskott-Aldrich syndrome (WAS), autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), autoimmune lymphoproliferative syndrome (ALPS) and idiopathic CD4(+) lymphocytopenia. Also we present findings from an adult cohort from the outpatient clinic of the Rheumatology Division of Universidade Federal de São Paulo. The PID manifestations found by our study group were considered mild in terms of severity of infections and mortality in early life. Thus, it is possible that some immunodeficiency states are compatible with survival regarding infectious susceptibility; however these states might represent a strong predisposing factor for the development of immune disorders like those observed in SLE.

PMID: 26239603 [PubMed – as supplied by publisher]

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Wiskott-Aldrich Syndrome: Description of a New Gene Mutation With Normal Platelet Volume.

J Pediatr Hematol Oncol. 2015 Aug 1;

Authors: Yoonessi L, Randhawa I, Nussbaum E, Saharti S, Do P, Chin T, Zwerdling T

Abstract
Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency characterized by an increased incidence of autoimmunity, malignancy, microthrombocytes with thrombocytopenia, eczema, and recurrent infections. In this case report, we present a novel mutation, hemizygous for c.1125_1129delTGGAC mutation in the WAS gene, and a unique clinical presentation. Our patient was initially diagnosed with a milk protein allergy after presenting with a lower gastrointestinal bleed, leukopenia, and thrombocytopenia with normal platelet volume. However, signs of vasculitis and detection of microthrombocytes required additional testing and consideration of WAS. This case report illustrates the importance of retaining a high index of clinical suspicion despite normal platelet volume, as well as adding to the growing number of known mutations associated with WAS.

PMID: 26241726 [PubMed – as supplied by publisher]

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